Amyotrophic lateral sclerosis (ALS) is a chronic progressive degenerative disease, which damages almost selectively the upper and lower motor neurons. The predominant symptom of ALS is loss of muscle strength due to degeneration of the motor neuron, but the sensory neuron is normal and a patient loses motor control but feels pain, and thus, ALS is a very cruel disease. A glutamate antagonist, riluzole has been solely used as a therapeutic agent, but a satisfactory effect cannot be attained. ALS is an incurable nervous disease, for which an effective therapeutic method cannot be found even now regardless of vigorous research activities for a long period of time, and urgent clarification of the mechanism of the onset and development of a therapeutic agent are required.